Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0262471 (ENT)
 5,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In summary, PGWG corresponds to an early phase of WG, presenting only with extravascular granuloma. Primarily, ENT, eye, or lung tissues are involved, but any organ may be affected. The diagnosis is made by typical palisading granuloma associated with elevated c-ANCA levels in the patients' serum. The concept of an early granulomatous lesion (PGWG) facilitates the early diagnosis of WG and leads to classification of different forms of disease according to the multistep evolution hypothesis: first PGWG, then localized or limited forms of the disease, and ultimately generalized vasculitis with renal involvement (classical WG). The concept of PGWG forms the basis for future therapeutic trials where folate antagonists are restricted to PGWG, while immunosuppressive treatment is required for the other stages of WG.
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PMID:Purely granulomatous Wegener's granulomatosis: a new concept for an old disease. 219 77

In a prospective multicentre study on the clinical significance of ANCA in renal diseases, sera from 920 patients with rapidly progressive renal failure and/or renal disease in association with extrarenal signs suggestive of a systemic vasculitis were tested for the presence of ANCA by indirect immunofluorescence (IIF) and ELISA. 193 of 920 cases (20.9%) were positive by IIF and 180 (19.5%) by ELISA, using a 'crude' cytoplasmic extract as substrate. The sensitivity and specificity of IIF for 'pauci-immune' cresentic necrotizing GN (CNGN), in association or not with systemic vasculitis, was 87.5 and 95.6% respectively. The IIF pattern and antigen specificity (alpha granules and MPO) correlated well with the clinical features: a cANCA pattern (alpha granules) was associated with ENT involvement (probable Wegener's granulomatosis); a pANCA pattern (MPO) with 'idiopathic' CNGN and small-vessel vasculitis without respiratory tract disease (microscopic polyarteritis); patients with a pulmonary-renal syndrome had either c or pANCA in a similar proportion. Our study confirms a high sensitivity and specificity of ANCA for patients with CNGN. ANCA should be considered an important diagnostic test in patients with renal diseases, especially in the presence of rapidly progressive renal failure.
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PMID:Diagnostic significance and antigen specificity of antineutrophil cytoplasmic antibodies in renal diseases. A prospective multicentre study. Italian Group of Renal Immunopathology. 752 6

In this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30 g/day over 5 days. Clinical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.
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PMID:Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin. 762 88

We reviewed 79 patients with clinically suspected Wegener's granulomatosis (WG) diagnosed in Nottingham between 1990 and 1997. Fifty-three patients were confirmed as having WG. We describe the symptoms and signs, features of diagnostic significance, ANCA results, biopsy sites, histology, treatment and outcome in this group. Nasal symptoms and signs had a positive predictive value of 63%, c-ANCA at presentation 100%, and positive nasal biopsy 100%. The negative predictive values were 41%; 79% and 74% respectively. From this study, we recommend that patients who have a negative ANCA and where there remains a clinical suspicion of WG, an ENT examination should be undertaken. Whilst a suspicious lesion should be biopsied and a positive histological picture has a 100% positive predictive value, a negative nasal biopsy does not exclude WG as 5 patients went on to develop a positive ANCA up to 4 years later. In 11 ANCA negative patients where there were signs of nasal mucosal disease, 6 had a positive biopsy and this highlights the importance of nasal biopsy. The c-ANCA should be repeated in patients with a negative c-ANCA and biopsy results at presentation, in whom there remains a clinical suspicion of WG.
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PMID:Wegener's granulomatosis--a review of diagnosis and treatment in 53 subjects. 992 63

The primary ANCA associated vasculitides, Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA), frequently affect the ENT region. For several reasons WG is of special significance for the otorhinolaryngologist. First, disease activity limited to the upper respiratory tract (localized WG) often proceeds the systemic vasculitis (generalized WG). The early diagnosis therefore has decisive consequences for stage adapted therapy. Second, in most cases (nearly 80%) WG is diagnosed histologically on biopsy specimens from the ENT region. During the initial phase of WG this is of diagnostic relevance, because at this stage the serologic parameters (acute-phase proteins) usually have a normal value and PR3-ANCA is (still) negative in 2/3 of the patients. Third, in many cases recurrences reveal increased activity in the ENT region, or start in this area. Clinically in most cases chronic rhinosinusitis with crusting and epistaxis is seen, sometimes with septal perforation and/or saddle nose. Apart from this there are often unclear middle ear symptoms with recurrent effusions and the inner ear is sometimes also affected. Laryngeal manifestations are typically located in the subglottic area and lead to subglottic stenosis. In the differential diagnosis, diseases in which epitheloid cell granulomas occur, such as sarcoidosis and TBC, need to be considered, but also foreign body granulomas and fungus diseases. Finally malignant tumours, especially malignant lymphomas, have to be ruled out.
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PMID:[Manifestations of primary vasculitis in the ENT region]. 1158 19

A 49 year old woman was admitted because of general poor health condition having lasted for 15 days and been associated for 6 months with sinusitis. During hospitalisation, she developed thrombosis of the retina central artery and signs of cerebral vasculitis. A diagnosis of Wegener disease was suggested based on one side on the presence of multiple excavated pulmonary macronodules and, on the other side, on the signs of cerebral vasculitis. No renal dysfunction was documented in our patient. The detection of serum antineutrophil antibodies (c-ANCA and anti-PR3) confirmed the diagnosis of Wegener disease without the need of surgical pulmonary biopsy. In addition to the typical ENT and pulmonary symptoms, this particular clinical presentation of unusual ocular and cerebral symptoms, reminds us that Wegener disease is a vasculitis that can give multisystemic disorders.
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PMID:[Case of the month. Wegener's disease]. 1264 92

A case of Wegener's granulomatosis in female aged 47 is described. Otological symptoms and signs connected with middle ear inflammation and masked mastoiditis about two months outstriped other signs particular from lower respiratory tract. The patient was surgically treated--antromastoidectomy was performed. After few days rapid worsening of patient's general state followed with hectic fever and inflammatory pulmonary changes. The patient was next treated in the Clinic of Pulmonary Diseases and Tuberculosis where the diagnosis of Wegener's granulomatosis was established on the basis of immunological findings (antibodies c-ANCA and PR3). The patient is treated from 9 months with cyclophosphamide and prednisone with improvement. The inability of ENT-surgeon in proper diagnosis and treatment in initial stage of the disease is stressed.
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PMID:[Otologic signs and symptoms as first manifestations of Wegener's granulomatosis with very severe clinical course--review of the literature and case report]. 1531 97

A 55-year-old male presented with left-sided otorrhoea, hearing loss and tinnitus of 3 months duration. On clinical examination polypoid tissue was seen prolapsing in the external ear canal. A CT scan of the mastoid cells and middle ear showed otomastoiditis with osteolysis. Oral antibiotic therapy and eardrops were started. When a facial nerve paresis appeared one month later, a mastoidectomy was performed. The mastoid cells and middle ear were filled with a connective tissue-like substance. Postoperative corticosteroids were administered. Despite the therapy the facial nerve problem aggravated and the patient developed severe parietotemporal headache, meningeal irritation and somnolence. The diagnosis of neurosarcoidosis was hypothesised. Blood analysis, including c-ANCA's, culture of the otorrhoea and biopsies of the connective tissue were inconclusive. A CT scan of the brain showed thickening of the left tentorium. A biopsy of the dura indicated a diagnosis of Wegener's granulomatosis. The patient was treated with immunosuppressive medication with satisfactory results.
B-ENT 2006
PMID:Wegener's granulomatosis presenting as otomastoiditis. A case report. 1667 40

Wegener's granulomatosis (WG) is rarely diagnosed during the reproductive years and uncommonly manifests for the first time during pregnancy. We report a case of de novo WG presenting at 30 weeks gestation with classical symptoms of WG (ENT, pulmonary). The diagnosis was confirmed by radiological, laboratory, and histological investigations. With a multidisciplinary approach, she had a successful vaginal delivery of a healthy baby. She was treated successfully by a combination of steroids, azathioprine and intravenous immunoglobulin in the active phase of disease for induction of remission and by azathioprine and steroids for maintenance of remission. The significant improvement in her symptoms allowed us to continue her pregnancy to 37 weeks when delivery was electively induced. Transplacental transmission of PR3-ANCA occurred but the neonate remained well. This case of de novo WG during pregnancy highlights the seriousness of this disease and the challenge in management of such patients.
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PMID:Wegener's granulomatosis occurring de novo during pregnancy. 1964 53

To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG), seven as microscopic polyangitis (MPA) and one as Churg-Strauss syndrome (SCS). The median age of the patients was 57.7 years and the sex-ratio (M/F) was 1.6. The visceral manifestations included kidneys (100% of patients), lungs (75%), ENT (52% of WG), and nervous system (57% of MPA). The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28%) compared to WG cases (12%). After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up.
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PMID:Clinical features and outcomes of ANCA-associated renal vasculitis. 2238 23


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